1. A unique clinical phenotype of primary sclerosing cholangitis associated with Crohn's disease
- Author
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Jelena Djordjevic, Emma L. Culver, John Halliday, Roger W. Chapman, Simon Travis, Barbara Braden, and M. Lust
- Subjects
Male ,endocrine system diseases ,medicine.medical_treatment ,Kaplan-Meier Estimate ,Liver transplantation ,Severity of Illness Index ,Inflammatory bowel disease ,Gastroenterology ,0302 clinical medicine ,Crohn Disease ,Child ,0303 health sciences ,Crohn's disease ,Primary sclerosing cholangitis ,Smoking ,digestive, oral, and skin physiology ,Crohns disease ,General Medicine ,Middle Aged ,Ulcerative colitis ,3. Good health ,Cell Transformation, Neoplastic ,Phenotype ,Disease Progression ,Female ,030211 gastroenterology & hepatology ,Adult ,medicine.medical_specialty ,Adolescent ,Cholangitis, Sclerosing ,digestive system ,Disease-Free Survival ,Statistics, Nonparametric ,Young Adult ,03 medical and health sciences ,Sex Factors ,Ileum ,Internal medicine ,medicine ,Humans ,Aged ,Proportional Hazards Models ,Retrospective Studies ,030304 developmental biology ,business.industry ,Case-control study ,Cancer ,medicine.disease ,digestive system diseases ,Case-Control Studies ,Concomitant ,business - Abstract
Background and aims: A distinct clinical phenotype has been demonstrated for ulcerative colitis with concomitant primary sclerosing cholangitis (PSC). The course and behaviour of Crohns disease (CD) with PSC has, in contrast, never been defined. We aimed to define the characteristics of patients with concomitant PSC and CD. Methods: The Oxford PSC and IBD databases were abstracted for: PSC subtype, date of diagnosis, symptom onset, smoking history, Mayo Clinic PSC score and outcomes (hepatic failure, liver transplantation, Montreal CD classification, treatment, cancer and death). Patients with PSC/CD were matched 1:2 to two control groups: one with PSC/UC and one with isolated CD. Results: 240 patients with PSC were identified; 32 (13%) with CD, 129 (54%) with co-existing UC, and 79 had PSC without IBD. For PSC/CD vs. CD controls, isolated ileal CD was less common (6% vs. 31%, p=0.03). Smoking was less common in PSC/CD (13% vs. 34%, p=0.045). No difference in the distribution of CD, or treatment required was observed. For PSC/CD vs. PSC/UC controls, more patients with PSC/CD were female (50% vs. 28%, p=0.021). 22% of PSC/CD patients had small duct PSC compared with 6% with PSC/UC, (p=0.038). Major event-free survival was prolonged in the PSC/CD group compared with PSC/UC, (Cox regression p=0.04). Conclusion: Unlike PSC/UC, patients with PSC/CD were as likely to be female as male, more commonly had small duct PSC and less commonly progressed to cancer, liver transplantation, or death. Compared to patients with isolated CD, patients with PSC/CD were less likely to smoke or have ileal disease. (C) 2011 European Crohns and Colitis Organisation. Published by Elsevier B.V. All rights reserved.
- Published
- 2012